CAJ | 학술논문

报告1例神经纤维瘤病Ⅰ型并发腹部多重血管畸形和右肾萎缩.患者男,26岁.全身皮肤散在分布咖啡斑26年,鼻部、躯干多发结节、肿块16年,血压升高5年.体格检查:血压220/130mmHg(1 mmHg=0.133 kPa),躯干、四肢可见10余处咖啡斑,呈棕色,深浅不一,直径2～10 cm.右鼻翼有一不规则肿块,躯干、四肢密布数百个黄豆、蚕豆大结节,部分呈暗褐色,质韧,互相不融合.数字减影血管造影检查提示:腹部多发血管畸形;双肾三维CT提示:右肾萎缩.免疫组化染色结果示S-100蛋白、波形蛋白(vimentin)阳性,核因子(NF)弱阳性.
보고1례신경섬유류병Ⅰ형병발복부다중혈관기형화우신위축.환자남,26세.전신피부산재분포가배반26년,비부、구간다발결절、종괴16년,혈압승고5년.체격검사:혈압220/130mmHg(1 mmHg=0.133 kPa),구간、사지가견10여처가배반,정종색,심천불일,직경2～10 cm.우비익유일불규칙종괴,구간、사지밀포수백개황두、잠두대결절,부분정암갈색,질인,호상불융합.수자감영혈관조영검사제시:복부다발혈관기형;쌍신삼유CT제시:우신위축.면역조화염색결과시S-100단백、파형단백(vimentin)양성,핵인자(NF)약양성.
A ease of type Ⅰ neurofibromatosis complicating multiple vascular malformation of abdomen and right kidney atrophy is reported.A 26-year-old male presented with a 26-year history of coffee-colored spots on his trunk,a 16-year history of multiple nodules,and secondly to a 5-year history of hypertension.Physical examination showed the blood pressure was 220/130 mmHg.There were about ten light or deep brown caf6-au-lait spots and hundreds of densely covered fuscous bean sized nodules on his limbs and trunk.Digital subtraction angiography disclosed multiple vascular malformation of abdomen.Computer-assisted tbmography disclosed fight kidney atrophy.Immunohistologic examinations revealed S-100,vimentin,NF were all positive.